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Exercise Induced Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Georgetown University, Washington, DC, 20007

Abstract

Introduction: Pulmonary arterial hypertension (PAH) is the most common cause of scleroderma related deaths. New medications for PAH patients make it necessary to identify patients at high risk factors for PAH. This study looks at the use of an exercise echocardiogram in identifying patients who may have PAH and may be candidates for early therapeutic intervention.

Methods: This study included 54 scleroderma patients with symptoms suggesting they were at risk for developing pulmonary hypertension including, dyspnea on exertion, diffusing capacity (DLCO) less than 60% predicted, FVC<70% predicted, FVC%/DLCO% ratio >1.6 or resting right ventricular systolic pressure (RVSP) >35mmHg. The exercise echocardiogram protocol involved the standard Bruce stress echocardiogram protocol with re-measurement of the RVSP within one minute of stopping exercise. A ‘positive' exercise test was defined as an increase of at least 20mmHg in the RVSP with exercise. A right heart catheterization with exercise was performed in those with a ‘positive' exercise test.

Results: he resting mean RVSP was 34.5mmHg which increased to 51.4mmHg with exercise; 44 % had at a ‘positive' exercise test which correlated with a low DLCO, high FVC%/DLCO% ratio (p<0.001), a positive anti-centromere antibody and RVSP >35mmHg (p<0.05). PAH was confirmed by right heart catheterization in 81% of patients: 19% at rest and 62% of patients with exercise.

Conclusions: Exercise induced pulmonary hypertension is a common finding in patients at high risk for PAH. This may be a sensitive way to identify patients with early PAH. Long term follow up and early treatment should be studied in these patients.